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J Neurooncol. 2015 Nov;125(2):401-10. doi: 10.1007/s11060-015-1930-y. Epub 2015 Sep 9.

Primary and secondary gliosarcomas: clinical, molecular and survival characteristics.

Author information

1
Department of Neurosurgery, Medical University of South Carolina, 96 Jonathan Lucas St, Charleston, SC, 29425, USA. cachia@musc.edu.
2
Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.
3
Department of Neurology, Baylor College of Medicine, 6550 Fannin St, Houston, TX, 77030, USA.
4
Department of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.
5
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, 6565 Fannin Street, Houston, TX, 77030, USA.
6
Department of Family Health, The University of Texas Health Science Center School of Nursing, 6901 Bertner Ave, Houston, TX, USA.
7
Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, Building 82, Bethesda, MD, 20892, USA.

Abstract

Gliosarcoma is classified by the World Health Organization as a variant of glioblastoma. These tumors exhibit biphasic histologic and immunophenotypic features, reflecting both glial and mesenchymal differentiation. Gliosarcomas can be further classified into primary (de novo) tumors, and secondary gliosarcomas, which are diagnosed at recurrence after a diagnosis of glioblastoma. Using a retrospective review, patients seen at MD Anderson Cancer Center between 2004 and 2014 with a pathology-confirmed diagnosis of gliosarcoma were identified. 34 patients with a diagnosis of gliosarcoma seen at the time of initial diagnosis or at recurrence were identified (24 primary gliosarcomas (PGS), 10 secondary gliosarcomas (SGS)). Molecular analysis performed on fourteen patients revealed a high incidence of TP53 mutations and, rarely, EGFR and IDH mutations. Median overall survival (OS) for all patients was 17.5 months from the diagnosis of gliosarcoma, with a progression free survival (PFS) of 6.4 months. Comparing PGS with SGS, the median OS was 24.7 and 8.95 months, respectively (from the time of sarcomatous transformation in the case of SGS). The median OS in SGS patients from the initial diagnosis of GB was 25 months, with a PFS of 10.7 months. Molecular analysis revealed a higher than expected rate of TP53 mutations in GS patients and, typical of primary glioblastoma, IDH mutations were uncommon. Though our data shows improved outcomes for both PGS and SGS when compared to the literature, this is most likely a reflection of selection bias of patients treated on clinical trials at a quaternary center.

KEYWORDS:

Gliosarcoma; IDH mutation; Primary gliosarcoma; Secondary gliosarcoma; TP53 mutation

PMID:
26354773
DOI:
10.1007/s11060-015-1930-y
[Indexed for MEDLINE]

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